Materiały źródłowe

• #1 Prevention of infection in children and adolescents with primary immunodeficiency disorders – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23393904/

  Primary Immunodeficiency diseases (PIDs) are a heterogenous group of inherited disorders that may involve one or multiple components of the immune system. PIDs are uncommon, chronic and severe disorders, in which patients cannot mount a sufficiently protective immune response, leading to an increased susceptibility to infections. This review addresses the current practices for the prevention of infection in children and adolescents with PIDs, particular covering immunisations and antimicrobial prophylaxis. […] Immunoglobulin prophylaxis is the mainstay of treatment for PIDs and provides passive protection. Prophylactic antimicrobials are efficacious in children and adolescents with predominant defects in primary T cell immunodeficiency diseases and phagocytic disorders, and also with predominant defects in antibody production. Prophylactic antibiotics are suggested for patients with antibody deficiency diseases if recurrent infections exceed three per year, if severe infections occur despite adequate immunoglobulin replacement and in hypogammaglobulinaemic patients who have bronchiectasis. Certain immunisations are effective in antibody deficiencies, T cell deficiencies, complement deficiencies and phagocytic disorders. […] The cornerstones of the prevention of infection in most PID patients are: antimicrobial prophylaxis, appropriate vaccination, immunoglobulin replacement, for the more severe cases, and regular ongoing follow-up.

• #1 Prevention of infections during primary immunodeficiency. – Research – Institut Pasteur

  https://research.pasteur.fr/en/publication/prevention-of-infections-during-primary-immunodeficiency/

  Because infectious diseases are a major source of morbidity and mortality in the majority of patients with primary immunodeficiencies (PIDs), the application of a prophylactic regimen is often necessary. […] To homogenize practices among centers, the French National Reference Center for PIDs aimed at elaborating recommendations for anti-infectious prophylaxis for the most common PIDs. […] Grading of prophylaxis was done using strength of recommendations (decreasing from A to D) and evidence level (decreasing from I to III). These might help infectious diseases specialists in the management of PIDs and improving the outcome of patients with PIDs.

• #1 Antibiotic prophylaxis in primary immune deficiency disorders – PubMed

  https://pubmed.ncbi.nlm.nih.gov/24565703/

  Long-term prophylactic antibiotics are being widely implemented as primary or adjunctive therapy in primary immune deficiencies. […] This review will provide evidence for the use of antibiotic prophylaxis in various primary immune deficiency populations, especially highlighting the role antibiotic prophylaxis in primary antibody deficiency syndromes. […] Overall, examination of available data on the use of prophylactic antibiotics in antibody deficiency syndromes merit future investigation in well-designed multicenter prospective trials because this population has few other management options.

• #1

  https://journals.lww.com/co-allergy/fulltext/2009/12000/antimicrobial_prophylaxis_for_primary.7.aspx

  Antibiotic prophylaxis is one of the mainstays of therapy of primary immunodeficiencies. We aim to summarize what is known about antibiotic prophylaxis for select primary immunodeficiencies. […] Currently, antibiotic prophylaxis is guided by the common microbial pathogens seen in specific immunodeficiencies, and experience with other chronic illnesses such as cystic fibrosis, HIV, and immunosuppression from transplantation. Controlled studies are necessary to address the preferred antimicrobial and immunomodulator regimens for most of the primary immunodeficiencies.

• #1 Inborn errors of immunity (primary immunodeficiencies) | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-024-00938-z

  Initial therapy for patients with SCID or other CID is supportive and involves aggressive treatment of the established infection, Ig replacement therapy, and antibiotic, antiviral, and/or antifungal prophylaxis to reduce the frequency and severity of infections. […] Patients with SCID should start prophylaxis against PJP. […] Since SCID is fatal unless the underlying defect is corrected, definitive therapy with HSCT should be initiated as quickly as possible. […] The mainstay of therapy for most antibody-deficiency disorders is intravenous (IV) or subcutaneous (SC) Ig replacement therapy and immunoprophylaxis with vaccines; in fact, many patients will require this treatment lifelong. […] For patients with recurrent infections, prophylactic antibiotic therapy (particularly with agents that provide coverage of Streptococcus pneumoniae and Haemophilus influenzae) may also be needed in addition to Ig replacement therapy.

• #1 American Academy of Allergy, Asthma & Immunology Annual Meeting

  https://www.healio.com/news/allergy-asthma/20240320/vaccination-prophylaxis-recommended-for-children-with-primary-immunodeficiencies

  Vaccination, prophylaxis recommended for children with primary immunodeficiencies. Children with primary immunodeficiencies should receive routine inactive vaccinations and other prophylactic treatment, according to a presentation at the American Academy of Allergy, Asthma Immunology Annual Meeting. Two doses of PPSV23 5 years apart are recommended. Antibiotics should be considered when upper respiratory infection symptoms appear. Physicians also should consider prophylactic antibiotics for patients who have recurrent bacterial sinopulmonary infections, with trimethoprim/sulfamethoxazole recommended in particular. When symptoms of upper respiratory infection develop, physicians should consider using antibiotics early. Finally, physicians should consider prophylaxis for mycobacterium avium complex with azithromycin for patients with congenital athymia, with or without 22q11.2 deletion syndrome. Starting them on azithromycin is very important.

• #1 Primary Immunodeficiencies | AAFP

  https://www.aafp.org/pubs/afp/issues/2003/1115/p2001.html

  Currently, more than 80 primary immunodeficiencies are recognized by the World Health Organization.1 While most of these disorders present in childhood, they can manifest later in life. Some primary immunodeficiencies, such as common variable immunodeficiency disorder, present in patients who are in their 20s or 30s. Patients with primary immunodeficiency disorders are susceptible to infections that, if left untreated, may be fatal. […] When recurrent infections are a problem, many patients with primary immunodeficiencies are managed with antibiotics alone or in combination with IVIG. For example, in patients with chronic granulomatous disease, prophylactic therapy with trimethoprim-sulfamethoxazole (Bactrim, Septra) reduces the incidence of severe infections by 50 percent.4 Similarly, treatment for complement deficiencies is directed at preventing infection, and consists of antibiotic prophylaxis and immunizations for encapsulated bacteria (e.g., heptovalent pneumococcal vaccine, Haemophilus b conjugate vaccine, meningococcal polysaccharide vaccine).14

• #1

  https://empendium.com/mcmtextbook/table/031_5548

  Avoidance of live vaccines (except SIGAD, THI) […] Antibiotic prophylaxis: All immunodeficiencies of this type if infections continue despite IVIG […] Avoidance of live vaccines: All immunodeficiencies of this type […] Avoidance of nonirradiated blood or products: All immunodeficiencies of this type […] Avoidance of CMV-positive blood or cells: All immunodeficiencies of this type […] Antibiotic prophylaxis: All immunodeficiencies of this type […] Antifungal prophylaxis: SCID, HIGM, HIES, ICD4L […] Avoidance of live vaccines: All immunodeficiencies of this type […] Antibiotic prophylaxis: All immunodeficiencies of this type […] Avoidance of live vaccines: All immunodeficiencies of this type […] Antibiotic prophylaxis: All immunodeficiencies of this type […] Avoidance of live vaccines: All immunodeficiencies of this type […] Antibiotic prophylaxis: All complement deficiencies.

• #1 ASCIA Inborn Errors of Immunity (Primary Immunodeficiencies) Clinical Care Standard – Australasian Society of Clinical Immunology and Allergy (ASCIA)

  https://www.allergy.org.au/hp/papers/ascia-iei-pid-clinical-care-standard

  People with IEI should have access to healthcare services that are equitable, accessible, timely, efficient, integrated, safe, effective and culturally appropriate. […] People with IEI should be supported by their medical team to learn about their condition, self-administration of therapies and effective self-care strategies, so that they are empowered to take an active role in managing their condition. […] When immunoglobulin therapy is indicated, it should be started as soon as it is clinically indicated, ideally within 4 weeks. […] Many people with IEI require ongoing appropriate antimicrobial therapy to reduce complications from recurrent and/or severe infections. This should be adequately funded and readily accessible. […] People with IEI are at increased risk of complications from infections and therefore require special consideration in healthcare settings (inpatient and outpatient), including the following infection control measures:

• #1 Update on the treatment of primary immunodeficiencies | Allergologia et Immunopathologia

  https://www.elsevier.es/en-revista-allergologia-et-immunopathologia-105-articulo-update-on-treatment-primary-immunodeficiencies-13110313

  The adverse effects of the IV infusion of gammaglobulin can be due to an excessively rapid administration to patients who receive it for the first time, patients with infection at the time of the infusion, or if more than four to six weeks have passed since the previous dose was administered. […] The monthly dose used is the same but is divided over four weeks. […] Comparative studies of the efficacy and safety of IVIg and SCIg for replacement therapy found no significant differences with respect to the number of infections or adverse reactions, and recent studies have shown that SCIg administered at home is associated with better quality of life than IVIg administered in the hospital. […] Prophylaxis: Non-absorbable oral antibiotics (colimycin, neomycin, vancomycin). Antifungal agents (fluconazole, amphotericin). Co-trimoxazole. Aciclovir-ganciclovir. […] Prevention: Selection of the best-matched donor possible. Immunossupression prior to SCT (calcineurin inhibitors: cyclosporine or mycophenolate).

• #1 Update on the treatment of primary immunodeficiencies | Allergologia et Immunopathologia

  https://www.elsevier.es/en-revista-allergologia-et-immunopathologia-105-articulo-update-on-treatment-primary-immunodeficiencies-13110313

  Update on the treatment of primary immunodeficiencies. […] Treatment with immunoglobulins is indicated in cases of humoral immunodeficiencies and in selected cases of combined immunodeficiencies. […] Immunoglobulins help to keep the patients free of symptoms and infections as these substances are able to neutralise infectious agents, modulate and promote the immune response and favour phagocytosis. […] Prophylactic immunossupression (as well as donor T lymphocyte depletion in haploidentical and unrelated donors) is performed to avoid or minimize GVHD. […] The patient must be maintained in a laminar flow room with broad anti-infectious prophylaxis and with the intravenous administration of gammaglobulin for a variable period. […] The recommended dose to avoid infections or hospitalisations and to improve lung function is 400 to 600 mg/kg, aiming to maintain an IgG level of over 500 mg/ml or 350 mg/ml over the baseline level.

• #1 When It Turns Out to Be Primary Immunodeficiency Disease: Benefits of Early Diagnosis

  https://www.nufactor.com/resources/ig/articles/ar-when-it-turns-out-to-be-primary-immunodeficiency-disease-benefits-of-early-diagnosis.html

  Early referral for workup of patients with any of more than 150 occult primary immunodeficiency diseases can dramatically reduce hospitalizations, permanent disability and high costs of care. […] It often takes years for infants born with a PIDD to be diagnosed and to start receiving immune globulin prophylaxis or other disease-appropriate therapy. […] For CVID and certain other disorders involving impaired antibody production, adequate replacement with IVIG or subcutaneous IG (SCIG) has been shown to reduce the incidence of pneumonia and prevent the progression of lung disease. […] After more than 30 years of experience, accumulating evidence has prompted recommendations to approach or exceed the lower limit of IgG concentration for normal healthy adults, which is approximately 700 mg/L.

• #1 Vaccine Recommendations for Primary Immunodeficiency Patients and Family Memberslogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na34767/2014/06/04/vaccine-recommendations-primary-immunodeficiency-patients

  The Medical Advisory Committee of the Immune Deficiency Foundation offers additional guidance on vaccinations to be avoided and encourages routine vaccination when appropriate. […] Live viral and bacterial vaccines can cause serious infections in primary immunodeficiency patients. […] Primary B-cell immunodeficiency (e.g., X-linked agammaglobulinemia and common variable immunodeficiency): Patients with severe deficiencies should avoid oral polio, yellow fever, smallpox, live attenuated influenza, and live bacterial (typhoid and BCG) vaccines. […] Primary T-cell deficiency before reconstitution (e.g. severe combined immunodeficiency and complete DiGeorge): Patients with severe deficiencies should avoid all live viral and bacterial vaccines (oral polio; yellow fever; measles, mumps, rubella (MMR); herpes zoster; smallpox; rotavirus; herpes zoster; live attenuated influenza; typhoid; BCG vaccines).

• #1 Approach to the Patient With Suspected Immunodeficiency – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/immunology-allergic-disorders/immunodeficiency-disorders/approach-to-the-patient-with-suspected-immunodeficiency

  Infection can be prevented by advising patients to avoid environmental exposures and not giving them live-virus vaccines (eg, varicella, rotavirus, measles, mumps, rubella, herpes zoster, yellow fever, oral polio, intranasal influenza vaccines) or BCG (bacille Calmette-Gurin). Pneumococcal, meningococcal, diphtheria-tetanus-pertussis (DTaP), hepatitis B, and Haemophilus influenzae type b (Hib) vaccines are the recommended risk-specific vaccines, but their effectiveness varies with the degree of immunodeficiency (1). […] Prophylaxis for reactivation of hepatitis B or hepatitis C in immunosuppressed patients with serologic evidence of exposure can be provided by antiviral medications such as lamivudine or entecavir. […] Patients at risk of serious infections (eg, those with SCID, chronic granulomatous disease, Wiskott-Aldrich syndrome, or asplenia) or of specific infections (eg, with Pneumocystis jirovecii in patients with T-cell disorders) can be given prophylactic antibiotics (eg, trimethoprim/sulfamethoxazole 5 mg/kg orally twice a day).

• #1 Vaccine Recommendations for Primary Immunodeficiency Patients and Family Memberslogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na34767/2014/06/04/vaccine-recommendations-primary-immunodeficiency-patients

  Complement deficiencies: Patients can receive all vaccines. […] White blood cell disorders (e.g., neutropenias, chronic granulomatous disease, and leukocyte adhesion defects): Patient can receive all routine vaccines but should avoid live bacterial vaccines (BCG and salmonella). […] Close contacts: Family members of immunodeficient patients can receive all vaccines except oral polio and small pox. […] Personal vaccination and herd immunity are especially important for patients with primary immunodeficiency. Such patients should be vaccinated as recommended above and family members should be encouraged to receive all vaccines except oral polio (rarely given in the U.S.).

• #1 Primary immunodeficiency // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/primary-immunodeficiency

  Because primary immune disorders are caused by genetic changes, there’s no way to prevent them. But when you or your child has a weakened immune system, you can take steps to prevent infections: […] Practice good hygiene. Wash your hands with mild soap after using the toilet and before eating. […] Take care of your teeth. Brush your teeth at least twice a day. […] Eat right. A healthy, balanced diet can help prevent infections. […] Be physically active. Staying fit is important to your overall health. Ask your doctor what activities are appropriate for you. […] Get enough sleep. Try to go to sleep and get up at the same time daily, and get the same number of hours of sleep every night. […] Manage stress. Some studies suggest that stress can hamper your immune system. Keep stress in check with massage, meditation, yoga, biofeedback or hobbies. Find what works for you. […] Avoid exposure. Stay away from people with colds or other infections and avoid crowds. […] Ask your doctor about vaccinations. Find out which ones you should have.

• #1 Pregnancies among patients with primary immunodeficiencies

  https://www.aaaai.org/tools-for-the-public/latest-research-summaries/the-journal-of-allergy-and-clinical-immunology/2023-the-journal-of-allergy-and-clinical-immunolog/pregnancy

  Although 89% of the women had experienced at least one manifestation of PID (infection, autoimmunity, lymphoproliferation, cancer) before pregnancy onset, PID was already diagnosed in only 49% of them. […] Only 57% of pregnancies were conducted with optimal anti-infectious prophylaxis. […] Pregnancies conducted with optimal prophylaxis had a better outcome than those without (OR 3.23 [IC95% 1.44-7.48], p=0.005). […] The study emphasizes the critical importance of offering to pregnant patients a multidisciplinary management in collaboration with an expert center and a tailored fit anti-infectious prophylaxis. […] In this article, authors propose recommendations for prophylaxis management during pregnancy.

• #1 When It Turns Out to Be Primary Immunodeficiency Disease: Benefits of Early Diagnosis

  https://www.nufactor.com/resources/ig/articles/ar-when-it-turns-out-to-be-primary-immunodeficiency-disease-benefits-of-early-diagnosis.html

  Beyond dosing to achieve a recommended target IgG trough level, one additional step in individualizing IgG replacement therapy is to adjust dosage upward, if and as needed, to minimize infection in that patient. […] The biggest problem that persists with PIDD remains the years-long delay in diagnosing these individuals in the first place. […] Clearly, the responsibility for earlier diagnosis of PIDD ultimately resides with each primary physician who interfaces with patients every day.

• #1

  https://link.springer.com/article/10.1007/s10875-020-00841-3

  Patients with IgSD SPAD often present with recurrent infections. Previous retrospective studies have shown that prophylactic antibiotics (PA) and immunoglobulin replacement therapy (IRT) can both be effective in preventing these infections; however, this has not been confirmed in a prospective study. […] Although there is no general consensus regarding the treatment of these patients, multiple sources have advocated a step-up approach for the prevention of infections. As a first-line treatment, additional vaccinations combined with increased vigilance and appropriate antibiotic therapy in the case of bacterial infections can lead to significant clinical improvement. In the absence of improvement, prophylactic antibiotics (PA) are often used to reduce the number of infections. Patients with persistent bacterial infections despite PA can be treated with IRT to further reduce the infectious burden.

• #1

  https://link.springer.com/article/10.1007/s10875-020-00841-3

  Open, non-placebo-controlled studies have shown that both PA and IRT can be effective in patients with IgSD and SPAD. […] The current study design, a randomized, controlled crossover trial, was selected as the optimal design to show differences in the efficacy of prophylactic treatment in a relatively rare disease where the cohort sizes are expected to be small. […] Overall, we found that overall, prophylactic antibiotics and IRT were equally efficient in preventing infections in a cohort of patients with IgSD SPAD. However, a subgroup of patients with persistent infections during treatment with PA showed a significant reduction in infections after switching to IRT.

• #1 Inborn errors of immunity (primary immunodeficiencies) | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-024-00938-z

  The management of innate disorders depends on the type of defect. For phagocyte disorders, therapy is primarily supportive and includes both antibiotic and antifungal prophylaxis. […] Management of these disorders focuses on antibiotic prophylaxis for the prevention of recurrent infections and vaccination.

• #1 Primary Immunodeficiencies | Immunopaedia

  https://www.immunopaedia.org.za/immunology/special-focus-area/1-primary-immunodeficiencies/

  Trimethoprim/sulfamethoxazole (TMP/SMX) prophylaxis has reduced the frequency of bacterial infections markedly. In patients receiving TMP/SMX prophylaxis, staphylococcal infections are essentially confined to the liver and cervical lymph nodes. […] In recent years, fungal infections, typically those due to Aspergillus species, have become more predominant. Although itraconazole prophylaxis has been shown to reduce fungal infection, newer antifungals, such as voriconazole and posaconazole, should further reduce fungal mortality in CGD. […] Therefore, current recommended prophylaxis in CGD is trimethoprim/sulfamethoxazole, itraconazole, and interferon gamma.

• #1 About Primary Immunodeficiency (PI) | Primary Immunodeficiency (PI) | CDC

  https://www.cdc.gov/primary-immunodeficiency/about/index.html

  Early diagnosis can help prevent or delay some of the health problems caused by PI. […] Taking steps to prevent infection is very important if you have PI. These steps include washing your hands the right way, taking good care of your teeth, maintaining healthy habits, including being physically active, eating healthy, and getting enough sleep, avoiding crowds and exposure to people who are sick, and asking your healthcare provider which vaccinations are safe for you. […] In some cases, people with PI cannot have live vaccines such as rotavirus, chickenpox, oral polio, and measles, mumps, rubella. Newborn screening for SCID can find babies with this PI early, before they receive these vaccines, so that they are not given these vaccines prior to treatment for SCID.

• #1 Evaluation of Primary Immunodeficiency Disease in Children | AAFP

  https://www.aafp.org/pubs/afp/issues/2013/0601/p773.html

  Physicians should suspect T-cell disorders and human immunodeficiency virus infection in children who have diarrhea, show failure to thrive, and have opportunistic infections. […] Physicians should suspect a primary immunodeficiency disease in children who have unusually severe and recurrent infections with common pathogens, or infections with unusual pathogens. […] When a primary immunodeficiency disease is suspected in a child, initial laboratory testing should include a human immunodeficiency virus test, complete blood count with differential, and measurement of serum immunoglobulin and complement levels. […] A basic laboratory workup that includes testing for human immunodeficiency virus (HIV) antibody, complete blood count with differential, and measurement of serum immunoglobulin and complement levels can identify children who need further testing and referral to a subspecialist for a suspected immunodeficiency disease.

• #1 Primary Immunodeficiency – Causes, Symptoms, and Treatments

  https://csipharmacy.com/understanding-pid/

  It is essential for individuals with PID to receive timely diagnosis and treatment to help manage the condition, reduce future complications due to recurrent infections, and improve quality of life. […] To help prevent or treat infections, individuals with PID may be prescribed long-term antibiotics. These antibiotics often provide much-needed support to the weakened immune system. […] While living with Primary Immunodeficiency can be challenging, many individuals with PID lead fulfilling lives with the right medical care. Early diagnosis, regular monitoring, and proper treatments are key to reducing the risk of infections and improving quality of life. […] Access to in-home IVIg and SCIg treatments from CSI Pharmacy provides a convenient and effective treatment option.

• #2 Primary immunodeficiency // Middlesex Health

  https://middlesexhealth.org/learning-center/diseases-and-conditions/primary-immunodeficiency

  Because primary immune disorders are caused by genetic changes, there’s no way to prevent them. But when you or your child has a weakened immune system, you can take steps to prevent infections: […] Practice good hygiene. Wash your hands with mild soap after using the toilet and before eating. […] Take care of your teeth. Brush your teeth at least twice a day. […] Eat right. A healthy, balanced diet can help prevent infections. […] Be physically active. Staying fit is important to your overall health. Ask your doctor what activities are appropriate for you. […] Get enough sleep. Try to go to sleep and get up at the same time daily, and get the same number of hours of sleep every night. […] Manage stress. Some studies suggest that stress can hamper your immune system. Keep stress in check with massage, meditation, yoga, biofeedback or hobbies. Find what works for you. […] Avoid exposure. Stay away from people with colds or other infections and avoid crowds. […] Ask your doctor about vaccinations. Find out which ones you should have.

• #2 Inborn errors of immunity (primary immunodeficiencies) | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-024-00938-z

  Primary immunodeficiencies (PID), now often referred to as inborn errors of immunity (IEI), are a large heterogeneous group of disorders that result from deficiencies in immune system development and/or function. […] Early consultation with a clinical immunologist is essential, as timely diagnosis and treatment are imperative for preventing significant disease-associated morbidity and mortality. […] The treatment of IEIs is complex and generally requires both supportive and definitive strategies, including but not limited to, immunoglobulin replacement therapy, antibiotic prophylaxis, immune response modifiers, and hematopoietic stem cell transplantation. […] Antibiotic and antifungal prophylaxis, and optimized immunization schedules are also recommended for many IEIs to prevent the frequency and severity of infections.

• #2 Prevention of infection in children and adolescents with primary immunodeficiency disorders – PubMed

  https://pubmed.ncbi.nlm.nih.gov/23393904/

  Primary Immunodeficiency diseases (PIDs) are a heterogenous group of inherited disorders that may involve one or multiple components of the immune system. PIDs are uncommon, chronic and severe disorders, in which patients cannot mount a sufficiently protective immune response, leading to an increased susceptibility to infections. This review addresses the current practices for the prevention of infection in children and adolescents with PIDs, particular covering immunisations and antimicrobial prophylaxis. […] Immunoglobulin prophylaxis is the mainstay of treatment for PIDs and provides passive protection. Prophylactic antimicrobials are efficacious in children and adolescents with predominant defects in primary T cell immunodeficiency diseases and phagocytic disorders, and also with predominant defects in antibody production. Prophylactic antibiotics are suggested for patients with antibody deficiency diseases if recurrent infections exceed three per year, if severe infections occur despite adequate immunoglobulin replacement and in hypogammaglobulinaemic patients who have bronchiectasis. Certain immunisations are effective in antibody deficiencies, T cell deficiencies, complement deficiencies and phagocytic disorders. […] The cornerstones of the prevention of infection in most PID patients are: antimicrobial prophylaxis, appropriate vaccination, immunoglobulin replacement, for the more severe cases, and regular ongoing follow-up.

• #2 Immunodeficiency Disorders Program | Rochester Regional Health

  https://www.rochesterregional.org/services/allergy-immunology-rheumatology/immunodeficiency-disorders-program

  Antibiotic prophylaxis. Antibiotics are typically given at low doses on a daily or every few day basis to minimize side effects and maximize infection prevention. Long-term prophylactic antibiotics have been widely implemented for years, improving clinical outcomes for several primary immunodeficiencies.

• #2 Primary Immunodeficiencies | Immunopaedia

  https://www.immunopaedia.org.za/immunology/special-focus-area/1-primary-immunodeficiencies/

  Trimethoprim/sulfamethoxazole (TMP/SMX) prophylaxis has reduced the frequency of bacterial infections markedly. In patients receiving TMP/SMX prophylaxis, staphylococcal infections are essentially confined to the liver and cervical lymph nodes. […] In recent years, fungal infections, typically those due to Aspergillus species, have become more predominant. Although itraconazole prophylaxis has been shown to reduce fungal infection, newer antifungals, such as voriconazole and posaconazole, should further reduce fungal mortality in CGD. […] Therefore, current recommended prophylaxis in CGD is trimethoprim/sulfamethoxazole, itraconazole, and interferon gamma.

• #2 Approach to the Patient With Suspected Immunodeficiency – Immunology; Allergic Disorders – Merck Manual Professional Edition

  https://www.merckmanuals.com/professional/immunology-allergic-disorders/immunodeficiency-disorders/approach-to-the-patient-with-suspected-immunodeficiency

  Infection can be prevented by advising patients to avoid environmental exposures and not giving them live-virus vaccines (eg, varicella, rotavirus, measles, mumps, rubella, herpes zoster, yellow fever, oral polio, intranasal influenza vaccines) or BCG (bacille Calmette-Gurin). Pneumococcal, meningococcal, diphtheria-tetanus-pertussis (DTaP), hepatitis B, and Haemophilus influenzae type b (Hib) vaccines are the recommended risk-specific vaccines, but their effectiveness varies with the degree of immunodeficiency (1). […] Prophylaxis for reactivation of hepatitis B or hepatitis C in immunosuppressed patients with serologic evidence of exposure can be provided by antiviral medications such as lamivudine or entecavir. […] Patients at risk of serious infections (eg, those with SCID, chronic granulomatous disease, Wiskott-Aldrich syndrome, or asplenia) or of specific infections (eg, with Pneumocystis jirovecii in patients with T-cell disorders) can be given prophylactic antibiotics (eg, trimethoprim/sulfamethoxazole 5 mg/kg orally twice a day).

• #2 Vaccine Recommendations for Primary Immunodeficiency Patients and Family Memberslogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na34767/2014/06/04/vaccine-recommendations-primary-immunodeficiency-patients

  The Medical Advisory Committee of the Immune Deficiency Foundation offers additional guidance on vaccinations to be avoided and encourages routine vaccination when appropriate. […] Live viral and bacterial vaccines can cause serious infections in primary immunodeficiency patients. […] Primary B-cell immunodeficiency (e.g., X-linked agammaglobulinemia and common variable immunodeficiency): Patients with severe deficiencies should avoid oral polio, yellow fever, smallpox, live attenuated influenza, and live bacterial (typhoid and BCG) vaccines. […] Primary T-cell deficiency before reconstitution (e.g. severe combined immunodeficiency and complete DiGeorge): Patients with severe deficiencies should avoid all live viral and bacterial vaccines (oral polio; yellow fever; measles, mumps, rubella (MMR); herpes zoster; smallpox; rotavirus; herpes zoster; live attenuated influenza; typhoid; BCG vaccines).

• #2 Vaccine Recommendations for Primary Immunodeficiency Patients and Family Memberslogo-32logo-40logo-60NEJM Journal WatchnejmJW_1L_RGB-b

  https://www.jwatch.org/na34767/2014/06/04/vaccine-recommendations-primary-immunodeficiency-patients

  Complement deficiencies: Patients can receive all vaccines. […] White blood cell disorders (e.g., neutropenias, chronic granulomatous disease, and leukocyte adhesion defects): Patient can receive all routine vaccines but should avoid live bacterial vaccines (BCG and salmonella). […] Close contacts: Family members of immunodeficient patients can receive all vaccines except oral polio and small pox. […] Personal vaccination and herd immunity are especially important for patients with primary immunodeficiency. Such patients should be vaccinated as recommended above and family members should be encouraged to receive all vaccines except oral polio (rarely given in the U.S.).

• #2 Primary Immunodeficiencies | AAFP

  https://www.aafp.org/pubs/afp/issues/2003/1115/p2001.html

  Most patients with primary immunodeficiencies should not receive live virus vaccines, including live oral poliovirus vaccine (OPV). Because of the risk of infection, OPV also should not be given to persons in close contact with these patients.14 In addition, most patients with primary immunodeficiencies should not receive measles, bacille Calmette-Gurin, and varicella vaccines. One exception would be patients with B-cell deficiency, who should receive varicella vaccine.

• #2 Primary immunodeficiency – Augusta HealthSearchClose SearchSearch IconSearch IconClose Search IconMobile Menu IconMobile Menu Close IconInstagramFacebookTwitterYoutube

  https://www.augustahealth.com/disease/primary-immunodeficiency/

  Because primary immune disorders are caused by genetic defects, there’s no way to prevent them. But when you or your child has a weakened immune system, you can take steps to prevent infections: […] Practice good hygiene. Wash your hands with mild soap after using the toilet and before eating. […] Take care of your teeth. Brush your teeth at least twice a day. […] Eat right. A healthy, balanced diet can help prevent infections. […] Be physically active. Staying fit is important to your overall health. Ask your doctor what activities are appropriate for you. […] Get enough sleep. Try to go to sleep and get up at the same time daily, and get the same number of hours of sleep every night. […] Manage stress. Some studies suggest that stress can hamper your immune system. Keep stress in check with massage, meditation, yoga, biofeedback or hobbies. Find what works for you. […] Avoid exposure. Stay away from people with colds or other infections and avoid crowds. […] Ask your doctor about vaccinations. Find out which ones you should have.

• #2 About Primary Immunodeficiency (PI) | Primary Immunodeficiency (PI) | CDC

  https://www.cdc.gov/primary-immunodeficiency/about/index.html

  Early diagnosis can help prevent or delay some of the health problems caused by PI. […] Taking steps to prevent infection is very important if you have PI. These steps include washing your hands the right way, taking good care of your teeth, maintaining healthy habits, including being physically active, eating healthy, and getting enough sleep, avoiding crowds and exposure to people who are sick, and asking your healthcare provider which vaccinations are safe for you. […] In some cases, people with PI cannot have live vaccines such as rotavirus, chickenpox, oral polio, and measles, mumps, rubella. Newborn screening for SCID can find babies with this PI early, before they receive these vaccines, so that they are not given these vaccines prior to treatment for SCID.

• #2 Primary immunodeficiency | Allergy, Asthma & Clinical Immunology | Full Text

  https://aacijournal.biomedcentral.com/articles/10.1186/s13223-018-0290-5

  The treatment of PIDs is complex and generally involves both supportive and definitive strategies. […] Initial therapy for patients with SCID or other CIDs is supportive and involves aggressive management of the established infection, Ig replacement therapy, and antibiotic and antifungal prophylaxis to reduce the frequency and severity of infections. […] Antibiotic and antifungal prophylaxis are also recommended for many PIDs to prevent the frequency and severity of infections.

• #2 Update on the treatment of primary immunodeficiencies | Allergologia et Immunopathologia

  https://www.elsevier.es/en-revista-allergologia-et-immunopathologia-105-articulo-update-on-treatment-primary-immunodeficiencies-13110313

  Update on the treatment of primary immunodeficiencies. […] Treatment with immunoglobulins is indicated in cases of humoral immunodeficiencies and in selected cases of combined immunodeficiencies. […] Immunoglobulins help to keep the patients free of symptoms and infections as these substances are able to neutralise infectious agents, modulate and promote the immune response and favour phagocytosis. […] Prophylactic immunossupression (as well as donor T lymphocyte depletion in haploidentical and unrelated donors) is performed to avoid or minimize GVHD. […] The patient must be maintained in a laminar flow room with broad anti-infectious prophylaxis and with the intravenous administration of gammaglobulin for a variable period. […] The recommended dose to avoid infections or hospitalisations and to improve lung function is 400 to 600 mg/kg, aiming to maintain an IgG level of over 500 mg/ml or 350 mg/ml over the baseline level.

• #2

  https://link.springer.com/article/10.1007/s10875-020-00841-3

  Patients with IgSD SPAD often present with recurrent infections. Previous retrospective studies have shown that prophylactic antibiotics (PA) and immunoglobulin replacement therapy (IRT) can both be effective in preventing these infections; however, this has not been confirmed in a prospective study. […] Although there is no general consensus regarding the treatment of these patients, multiple sources have advocated a step-up approach for the prevention of infections. As a first-line treatment, additional vaccinations combined with increased vigilance and appropriate antibiotic therapy in the case of bacterial infections can lead to significant clinical improvement. In the absence of improvement, prophylactic antibiotics (PA) are often used to reduce the number of infections. Patients with persistent bacterial infections despite PA can be treated with IRT to further reduce the infectious burden.

• #2 Evaluation of Primary Immunodeficiency Disease in Children | AAFP

  https://www.aafp.org/pubs/afp/issues/2013/0601/p773.html

  HIV infection should be considered in newborns and adolescents who present with diarrhea, failure to thrive, and unusual opportunistic infections. […] A complete blood count with differential should be obtained to screen for a T-cell or phagocytic disorder. […] Patients with B-cell disorders have low serum immunoglobulin levels and decreased production or response of immunoglobulins to vaccination. […] Complement disorders are screened by checking the components of the classic and alternative pathways. […] In 2010, the U.S. Department of Health and Human Services recommended routine screening for SCID in newborns.

• #2 ASCIA Inborn Errors of Immunity (Primary Immunodeficiencies) Clinical Care Standard – Australasian Society of Clinical Immunology and Allergy (ASCIA)

  https://www.allergy.org.au/hp/papers/ascia-iei-pid-clinical-care-standard

  Adherence to local infection control procedures and additional measures to mitigate risk of any respiratory infections, not limited to influenza, COVID and tuberculosis. […] People with IEI should be empowered by their clinical and allied health teams to make use of the information, educational resources and support services available through professional and patient/carer organisations. […] The patients clinical and multidisciplinary team members should empower, educate and support people with IEI (and carer/s or family members if applicable) to appropriately engage in managing their condition to optimise health outcomes. […] Young people with IEI should be supported by their clinical team and their carers to learn about their condition, self-administer therapies and self-care strategies, to be empowered and take an active role in managing their condition.

• #3

  https://journals.lww.com/co-allergy/fulltext/2009/12000/antimicrobial_prophylaxis_for_primary.7.aspx

  Antibiotic prophylaxis is one of the mainstays of therapy of primary immunodeficiencies. We aim to summarize what is known about antibiotic prophylaxis for select primary immunodeficiencies. […] Currently, antibiotic prophylaxis is guided by the common microbial pathogens seen in specific immunodeficiencies, and experience with other chronic illnesses such as cystic fibrosis, HIV, and immunosuppression from transplantation. Controlled studies are necessary to address the preferred antimicrobial and immunomodulator regimens for most of the primary immunodeficiencies.

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